Diltiazem ameliorated prolonged QTc intervals in HCM-affected family members with an ACTN2 mutation, translating hiPSC disease modeling to personalized clinical therapy.
channel blocker diltiazem reduced force amplitude, relaxation, and action potential duration to a greater extent in HCM than in isogenic control. We translated our findings to patient care and showed that diltiazem application ameliorated the prolonged QTc interval in HCM-affected son and sister of the index patient. These data provide evidence for this ACTN2 mutation to be disease-causing in cardiomyocytes, guiding clinical therapy in this HCM family. This study may serve as a proof-of-principle for the use of hiPSC for personalized treatment of cardiomyopathies.
Prondzynski et al. (Sun,) conducted a other in Hypertrophic cardiomyopathy. Diltiazem vs. Isogenic control was evaluated on QTc interval and cardiomyocyte force amplitude, relaxation, and action potential duration. Diltiazem ameliorated prolonged QTc intervals in HCM-affected family members with an ACTN2 mutation, translating hiPSC disease modeling to personalized clinical therapy.
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